#NASPGHAN19 Liver Symposium (Part 3)

Although I was unable to attend this year’s liver symposium at NASPGHAN19, I reviewed the lecture notes.  There is some terrific content.  Here are some of the slides (borrowed with permission from NASPGHAN).

Link to complete NASPGHAN Chronic Liver Disease Symposium 2019

SESSION III – UPDATE ON PORTAL HTN: ASSESSMENT AND MANAGEMENT

What do I do now? The management of portal hypertensive complications: Varices, ascites, and encephalopathy Rene Romero, MD, Children’s Hospital of Atlanta

When there is good function, but the flow is all wrong: Approach to non-cirrhotic portal hypertension Evelyn Hsu, MD, Seattle Children’s Hospital

The role of the interventional radiologist in the treatment of portal HTN: How can I help you?  Jared R. Green, MD, Ann and Robert H. Lurie Children’s Hospital (SLIDES NOT AVAILABLE)

When to consider surgery in the treatment of portal HTN?  Riccardo Superina, MD, FRCS(C), FACS, Northwestern University  (SLIDES NOT AVAILABLE)

What happens with 98,500 IU/day of Vitamin A


An interesting case report (LA Beste et al. NEJM 2016; 374: 73-8) reviews the presentation of a previously healthy 54 year old with ascites.  He initially indicated that he was taking 100 IU per day of vitamin A (his current dose), but later on directed questioning admitted that he had averaged 98,500 IU/day for prior 6 months.

The clinical-problem solving case reviews useful pointers about portal hypertension and in particularly noncirrhotic portal hypertension.  Vitamin A is a rare cause of noncirrhotic portal hypertension.

Other causes of noncirrhotic portal hypertension:

  • Prehepatic level: portal vein or splenic vein thrombosis, splanchnic arteriovenous malformation
  • Intrahepatic level: hepatic vasculitis, HIV infection, infiltrative disease, and medications
  • Posthepatic: Budd-Chiari syndrome, IVC obstruction, restrictive cardiac disease.
  • Worldwide, schistosomiasis is the most common reason.
  • When other causes have been excluded, idiopathic noncirrhotic portal hypertension may be diagnosed, “especially in patients with chronic infection, thrombophilia, and immunologic conditions such as SLE.”  In one series of 69 patients, the diagnosis of idiopathic noncirrhotic portal hypertension was delayed for more than a year in 25% of cases and 7% received an erroneous diagnosis of cryptogenic cirrhosis.

Other points:

  • When ascites is due to cirrhosis, other signs of liver disease are typically present, including jaundice and laboratory findings (low albumin, coagulopathy, hyperbilirubinemia) as well as absence of cirrhosis on biopsy.
  • Serum retinol levels poorly reflect total body stores of vitamin A (& was normal in this patient)
  • Vitamin A supplementation in appropriate doses can prevent blindness in areas where food stores are not secure.  But, consuming excessive doses can lead to being a case report.

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Mural, Old town San Juan

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Liver Update: Headlines and Links Only

  1. From AGA: Hepatic failure flagged as unexpected boceprevir safety signal in adverse event review. GI & Hep News: http://ow.ly/rSCEF 
  2. From NY Times: Spike in Harm to Liver Is Tied to Dietary Aids nyti.ms/JPN9fK 
  3. From Jeff Schwimmer (The Liver Post): First case report of Liver Cancer in a child with Nonalcoholic Fatty Liver Disease. He is only 7 years-old. http://goo.gl/6dJbzs 
  4. “Recurrence of Hepatopulmonary Syndrome Post-Orthotopic Liver Transplantation in a Patient with Noncirrhotic Portal Hypertension” Hepatology 2013; 58: 2205-06.
  5. “Management of Hepatitis B: Our Practice and How It Relates to the Guidelines” Clin Gastroenterol Hepatol 2014; 12: 16-26.  Terrific review and insights.
  6. “Acute Liver Failure” NEJM 2013; 369: 2525-34.
  7. “Cesarean Section Reduces Perinatal Transmission of Hepatitis B Virus Infection from Hepatitis B Surface Antigen-Positive Women to Their Infants” Clin Gastroenterol Hepatol 2013; 11: 1349-55. Retrospective, nonrandomized study -“performing elective cesarean section only in highly viremic mothers with pre-delivery HBV DNA levels ≥1,000,000 copies/mL may be advisable.”

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